Invited. Takayasu arteritis: MR manifestations and diagnosis of acute and chronic phase

Diagnosis of Takayasu arteritis is difficult because the clinical features are similar to those of other diseases. In the late occlusive or pulseless phase Takayasu arteritis, angiography usually demonstrates luminal changes such as such as stenosis, occlusion, or aneurysmal dilatation of the aorta and pulmonary artery and of their branches. However, absence of such luminal changes does not exclude the possibility of early or systemic phase Takayasu arteritis. Cross-sectional scanning such as CT scan and MRI plays an important role in demonstrating arterial wall changes in the early diagnosis of Takayasu arteritis. Improvement in the clinical findings and subsidence of the active inflammatory process can be expected with early steroid treatment. The common and uncommon MR appearances of the late occlusive phase and the recently described radiographic features of the early systemic phase are illustrated.     

Role of magnetic resonance imaging in the early diagnosis of Takayasu arteritis

Takayasu arteritis is a non-specific inflammatory process of unknown aetiology affecting the aorta and its major branches. It may lead to stenosis or occlusion. We have examined eight patients with clinically diagnosed Takayasu arteritis using contrast-enhanced MRI and also 3-D MR angiography. All patients were female and their ages varied between 22 and 48 years. We were able to show subtle arteritic changes and stenotic lesions in branch vessels in the early phase of the disease by using this imaging modality. Thus, we can state that contrast-enhanced MRI with 3-D MR angiography can be used for the initial diagnosis of Takayasu arteritis. It provides a means to evaluate the vascular tree non-invasively and may replace conventional angiography.     

Takayasu arteritis: diagnosis with breath-hold contrast-enhanced three-dimensional MR angiography

The purpose of the study was to determine the diagnostic accuracy of breath-hold contrast-enhanced three-dimensional (3D) magnetic resonance (MR) angiography in Takayasu arteritis. Thirty patients suspected of having Takayasu arteritis were examined with MR angiography and conventional angiography. Takayasu arteritis was diagnosed in 20 of these patients. MR angiography was performed using a 1.5-T system after bolus injection of 0.1 mmol/kg of gadodiamide. MR angiography clearly depicted various vascular lesions in the aorta and its major branches in all 20 patients with Takayasu arteritis. It also depicted pulmonary artery lesions in 10 (50%) of the 20 patients. MR angiography accurately depicted 323 (98%) of 330 arteries, but 7 (2%) stenotic arteries were overestimated as occluded. The sensitivity and specificity of MR angiography for the diagnosis of Takayasu arteritis were both 100%. Breath-hold contrast-enhanced 3D MR angiography clearly depicts various vascular lesions in both the systemic and pulmonary arteries in Takayasu arteritis, thus allowing a definitive diagnosis of Takayasu arteritis.     

大动脉炎血管壁的磁共振表现特点

目的：分析大动脉炎血管壁的MR表现特征及其临床应用价值。方法：32例临床确诊为大动脉炎的患者接受了MR检查,其中处于病情活动期20例,非活动期12例。分析并比较了两组患者受累动脉管壁的MR表现特征。结果：所有受累动脉的血管壁均不同程度的环形增厚。活动期组患者的管壁厚度较非活动期组患者明显增厚[（4．6±0．4）mm vs（3．4±0．6）mm,P〈0．01];比较两组增厚的血管壁形态,管壁呈“多环征”（分别为89／100和25／60）、内层明显强化（分别为66／100和24／60）、血管外界模糊不清（分别为72／100和20／60）等征象,经Х^2检验差异均有统计学意义（P〈0．01）。结论：大动脉炎血管壁的MR主要征象是管壁增厚及其信号改变。MRI能观察血管壁精细的形态学变化,对大动脉炎管腔狭窄前的早期诊断以及活动性判断具有重要价值。     

3.0 T高分辨率MR血管壁成像对大动脉炎活动性判断的价值

目的 探讨3.0 T高分辨率MR血管壁成像对大动脉炎活动性判断的价值.方法 26例确诊为大动脉炎的患者(根据Lupi-Herrea分类,为Ⅰ和Ⅲ型,均累及主动脉弓及其主要大分支)接受了颈部及上胸部MR血管壁成像,其中处于病情活动期16例,非活动期10例.分析并比较了2组患者受累动脉管壁的MRI表现特征.采用X~2检验对2组患者的血管壁形态进行统计学分析.结果 所有受累动脉的血管壁均有不同程度的环形增厚.活动期组患者的管壁厚度较非活动期组患者增厚明显[分别为(3.8±0.2)和(2.5±0.8) mm];比较2组增厚的血管壁形态,管壁呈多环征(分别为75/80和18/50)、内壁明显强化(分别为50/80和19/50)、血管外界模糊不清(分别为55/80和18/50)等征象,差异均有统计学意义(X~2值分别为50.39、7.41、13.40,P值均<0.01).结论 大动脉炎高分辨率MR血管壁成像的主要征象是管壁增厚及信号改变,3.0 T MR血管壁成像能精细观察血管壁的形态学变化,对大动脉炎的活动性判断具有重要价值.     

The thoracic aorta studied by MR imaging

Thirty-three patients with a variety of disorders of the thoracic aorta (aneurysm, dissection, Marfan syndrome, coarctation/pseudocoarctation, L-transposition, and Takayasu disease) were evaluated with magnetic resonance (MR) imaging. MR imaging delineated the presence and extent of thoracic aortic aneurysms and showed the relationship of the aneurysm to arch vessels; it also demonstrated intimal flaps and individual lumina in types A and B aortic dissection. Dilation of the ascending aorta in Marfan syndrome and focal narrowing of the aorta in coarctation were well visualized. The anteroposterior and side-to-side relationships of the aorta and pulmonary artery in L-transposition were demonstrated, as were aortic wall thickening and branch vessel narrowing in Takayasu arteritis. Initial experience suggests that MR imaging may provide a noninvasive method for evaluating thoracic aortic disease. Limitations include inferior spatial resolution, occasional difficulty in imaging the entire region of interest in one section, lack of signal from calcifications, and inability to monitor critically ill patients.     

Takayasu's arteritis: assessment of disease activity with contrast-enhanced MR imaging

OBJECTIVE: The purpose of this study was to evaluate the role of contrast-enhanced MR imaging in the determination of disease activity in patients with Takayasu's arteritis. SUBJECTS AND METHODS: High-resolution contrast-enhanced T1-weighted spinecho MR imaging using small fields of view (14-20 cm) and thin slices (4-5 mm) was performed in 26 patients with Takayasu's arteritis and 16 healthy subjects. The degree of aortic mural enhancement was assessed by measuring signal intensity and by visually estimating it in comparison with that of the myocardium. RESULTS: Contrast-enhanced MR imaging showed more enhancement of thickened aortic wall compared with myocardium, thus suggesting active Takayasu's arteritis on MR imaging in 16 patients. Determination of disease activity using contrast-enhanced MR imaging was concordant with clinical findings in 23 patients (88.5%). Contrast-enhanced MR findings were concordant with laboratory findings in most patients (erythrocyte sedimentation rate in 92.3% [24/26] and C-reactive protein in 84.6% [22/26]). The measured signal intensity of the aortic wall relative to that of myocardium during the early phase of contrast-enhanced MR imaging correlated well with the erythrocyte sedimentation rate (r = 0.78, p < 0.005) and with the C-reactive protein level (r = 0.63, p < 0.005). CONCLUSION: Contrast-enhanced MR imaging provides information about disease activity of Takayasu's arteritis, which may be useful in the diagnosis and treatment of Takayasu's arteritis.     

Three‐dimensional velocity mapping of thoracic aorta and supra‐aortic arteries in takayasu arteritis

Takayasu arteritis is an inflammatory disease of unknown etiology that involves the aorta, its major branches, and the pulmonary artery. We describe three patients with Takayasu arteritis who showed abnormal velocity profile of the thoracic aorta and supra‐aortic arteries on time‐resolved three‐dimensional (3D) phase‐contrast MR imaging and velocity mapping techniques. Compared with two comparative subjects, velocity reduction was observed in these arteries. The velocity reduction was prominent along the thickened arterial wall, even with normal luminal caliber, and the highest velocity was observed on the contralateral side. In one patient, the arterial flow velocity and its profile at systole were partly improved after the treatment. The time‐resolved 3D velocity mapping visualized the changes in the blood velocity profile at systole in Takayasu arteritis. J. Magn. Reson. Imaging 2010;31:1481–1485. ©2010 Wiley‐Liss, Inc.     

Value of F-18 FDG hybrid camera PET and MRI in early takayasu aortitis

Takayasu aortitis (TA) is a chronic inflammatory and fibrotic vasculitis of large- and medium-sized arteries. Early stages of the disease show a panarteritis and inflammatory wall thickening of the aorta and its branches, whereas advanced (fibrotic) stages comprise stenosis, aneurismatic transformation and occlusion. Magnetic resonance imaging visualises early-stage disease with high accuracy and is considered to be the method of choice in the diagnosis of TA. The aim of this article is the detailed comparison of FDG-PET performed with a hybrid camera and MR imaging in five patients with early TA. Five patients (median age 60 years) were enrolled during an ongoing prospective study on [18F]2'-deoxy-2-fluoro-D-glucose (FDG) hybrid camera PET in patients with fever of unknown origin (FUO). These patients underwent MR imaging after establishing the diagnosis of TA. Abnormal FDG uptake in the wall of the aorta was noted in all patients. The bracheocephalic artery and the common carotid arteries were visualized in 3 cases. Increased uptake of the subclavian artery was found in 3 patients and in 4 patients pathological uptake was noted in the ilio-femoral vessels. Of 34 vascular regions studied, 26 (76%) showed elevated FDG uptake. On transversal MR images vessel wall thickening and contrast enhancement of the thoracic aorta was found in 4 patients (ascending aorta/aortic arch: n=2; descending aorta: n=3; abdominal aorta: n=1). Additionally, vessel wall pathologies of the subclavian and the common carotid arteries could be shown in 1 patient and in another patient in the ilio-femoral arteries. No abnormalities were found using contrast-enhanced MR angiography. Of 28 vascular regions studied, 9 (32%) showed vasculitis on MRI. The FDG-PET is a suitable whole-body screening method in the primary diagnosis of early TA, especially in those cases with early disease that present with uncharacteristic symptoms such as FUO. Both MRI and MRA remain indispensable in the exact determination of the pathomorphological changes and in the documentation of complications such as stenosis, aneurismatic transformation and occlusion. Electronic Publication     

Takayasu arteritis: imaging spectrum at multidetector CT angiography

Takayasu arteritis is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its major branches and the pulmonary and coronary arteries. The non-specific inflammation of involved vessels usually leads to concentric wall thickening, fibrosis and thrombus formation. Diseased arteries become stenotic or occluded, undergo vascular remodelling or develop aneurysms. According to the involvement of arteries, six types of Takayasu arteritis are documented. The purpose of this pictorial review is to illustrate the various multidetector CT angiography appearances of Takayasu arteritis and to discuss the differential diagnosis.Takayasu arteritis (TA) is a chronic, idiopathic, inflammatory disease that primarily affects large vessels, such as the aorta and its major branches, pulmonary and coronary arteries. Because of considerable morbidity and mortality, accurate and early diagnosis plays a crucial role in improving the outcomes for patients with TA [1]. Unfortunately, the non-specific clinical presentations and laboratory test results frequently contribute to late diagnosis and delayed treatment [1]. Since large-artery biopsies cannot easily be done, imaging examination is essential for providing the diagnosis and differential diagnoses in patients with suspected TA.Conventional angiography has been traditionally considered the gold standard for the diagnosis of TA [2]. However, multidetector CT angiography (CTA) is emerging as a reliable tool in non-invasively depicting both luminal and mural lesions in the aorta and its main branches, which may facilitate the detection of vasculitis during the early phase of TA.In this article, we will review the CTA findings in TA and how this non-invasive method impacts patient care.     

Takayasu's arteritis: evaluation with three-dimensional time-of-flight MR angiography

Three-dimensional time-of-flight MR angiography was performed in 16 patients with Takayasu's arteritis. Two regions were evaluated, the arch of aorta and its intrathoracic major branches, and the abdominal aorta with proximal portions of its major visceral and renal branches. Individual arteries and aortic segments, i. e. aortic arch and abdominal aorta, were evaluated for abnormalities such as stenosis, occlusion, dilatation and aneurysm formation. The results were compared with contrast angiography. Follow-up MR angiography was performed in three patients after 9–12 months. MR angiography demonstrated steno-occlusive lesions in all the patients and aneurysms in 2. In comparison with contrast angiography, good correlation was found in 129 of the 145 arteries and aortic segments. For the 12 false-positive results, incorrect slab placement and overestimation of stenosis were implicated. Interestingly, there were three false-negative results and one occlusion was underestimated as stenosis. A new lesion developed in 1 patient and one stenosis progressed in another patient upon follow-up. Three-dimensional time-of-flight MR angiography is a simple and fairly accurate method for documenting the lesions in Takayasu's arteritis and for its follow-up. Received 12 April 1995; Revision received 30 August 1995; Accepted 15 September 1995     

Digital subtraction angiography in Takayasu arteritis

The usefulness and limitation of digital subtraction angiography (DSA) in Takayasu arteritis were investigated in 32 patients. Intravenous DSA was particularly useful in the follow-up of patients with an established diagnosis of Takayasu arteritis. Pulmonary arterial involvement could also be demonstrated with intravenous DSA as obstructive arterial changes and lack of accumulation of contrast medium in the pulmonary parenchyma. Detailed information concerning the site and extent of vascular involvement and development of collateral vessels were obtained with intraarterial DSA. Thickening of the thoracic aortic wall, however, could not be recognized with either intravenous or intraarterial DSA. Conventional angiography of the descending thoracic aortal is still required as an initial examination, particularly when there is no aortic arch involvement. However, there is no doubt that DSA has the potential to become the diagnostic procedure of choice in Takayasu arteritis.     

电子束CT诊断累及升主动脉及主动脉瓣的大动脉炎

目的 探讨电子束CT（EBCT）诊断累及升主动脉 及主动脉瓣的大动脉炎的临床价值。方法 自1996年4月至1999年9月,25例大动脉炎患者接受了EBCT检查。采用美国Imatron公司150－XP型EBCT扫描机,扫描方式：采用增强单层容积扫描（SSM）,扫描层厚3mm,扫描时间0.1s,心电门控;连续容积扫描（CVS）,层厚3mm,扫描时间0.1s.非离子型对比剂,300mgI/ml.其中17例累及升主动脉及主动脉瓣,4例因重度主动脉瓣关闭不全行换瓣手术,病理诊断大动脉炎。2例2年后因瓣周漏再次行换瓣手术。结果 17例均有升主动脉壁不同程度的管理增厚且延续至主动脉瓣水平;活动期10例动脉管壁增厚呈环状高密度影或分层状,内壁光滑。非活动期7例管壁增厚程度轻,呈低密度环。11例升主动脉扩张,左心室扩大,主动脉瓣中－重度关闭不全。4例手术换患者病理所见符合大动脉炎所见。结论 大动脉炎基本CT征象是动脉壁增厚,累及升主动脉者可同时累及主动脉瓣,造成主动脉瓣关闭不全,活动期应为手术禁忌征。EBCT对检出此类大动脉炎有重要价值并且可反映大动脉炎的过程。     

Sudden death due to Takayasu arteritis complication associated with situs inversus totalis: A case discovered at autopsy

Takayasu arteritis is a rare pathology that usually has general and atypical signs that make its diagnosis difficult. These characteristics can delay diagnosis, thus leading to complications and death. We, herein, report an autopsy case of a 25-year-old female patient with a history of multiple consultations for dyspnea. During these consultations, no diagnosis was made. She was found unconscious near her home and shortly after, she was declared dead. Forensic autopsy revealed superficial traumatic lesions. Internal examination revealed complete situs inversus. Multiple bilateral pleural adhesions and bilateral moderate effusion were found. The heart was heavy with thickening of the aortic wall (1.1cm), carotid arteries, and pulmonary trunk, associated with a large aortic valve and evidence of leakage. Histological examination of the aorta and its major branches showed features of panarteritis with segmental involvement. The vascular wall was thick with lymphoplasmacytic infiltrate and giant cells involving mainly the medio-adventitial junction. Disruption of the elastic lamina and reactive fibrosis in the intima were also noted. Diagnosis of large vessel vasculitis and particularly Takayasu arteritis was made.Death was therefore attributed to heart failure due to aortic insufficiency as a complication of Takayasu arteritis.     

Patología aórtica no urgente: diagnóstico clínico-radiológico de la aortitis

Aortitis is a pathological term designating inflammation of the aortic wall, regardless of its cause. The clinical presentation of aortitis is nonspecific and variable. Symptoms include abdominal pain, fever, and weight loss; acute phase reactants may also be elevated. Aortitis can be caused by a wide spectrum of entities, including from infectious processes to autoimmune diseases (Takayasu arteritis and giant cell arteritis are among the most common of these causing aortitis), and the prognosis and treatment of these entities vary widely. Various imaging techniques can be used to evaluate the lumen and wall of the aorta (such as multidetector computed tomography, magnetic resonance imaging, angiography, or PET-CT). This review focuses on the most common diseases that cause aortitis and on the clinical and radiological findings that are most useful for diagnosing and treating this condition appropriately.     

MR angiography of Takayasu arteritis.

We describe MR angiographic findings utilizing a three-dimensional time-of-flight technique and compare the results with angiography in a case of suspected Takayasu arteritis involving vessels to the neck and upper extremities.     

Follow-up of patients with previous treatment for coarctation of the thoracic aorta: comparison between contrast-enhanced MR angiography and fast spin-echo MR imaging

Regular follow-up is required in patients with previous intervention for coarctation of the aorta to detect recoarctation or aneurysm formation. In this study we describe the findings encountered on routine follow-up exams and we compare the use of contrast-enhanced 3D MR angiography (CE MRA) with fast spin-echo MRI (FSE) to study the thoracic aorta after previous intervention. In 51 consecutive patients previously treated for aortic coarctation, 74 MR studies of the thoracic aorta were performed during a 2-year period using CE MRA and FSE MRI. The thoracic aorta was evaluated for abnormalities of course, caliber, shape, and pathology of side branches. The CE MRA and FSE MRI studies were evaluated side by side by consensus of two reviewers evaluating which MR technique depicted the abnormalities of the thoracic aorta the best. Of 74 exams, six clinically important abnormalities were found: four aneurysms and two restenoses. Two small pseudoaneurysms were missed on the FSE studies. Contrast-enhanced MRA was judged to visualize aortic abnormalities better than FSE (47 of 74 MR studies) especially for the transverse aortic arch, coarctation site, left subclavian artery, and aortic arch configuration. For the ascending aorta and distal descending aorta, CE MRA and FSE performed equally well. Aortic diameters measured at four levels in the first 18 MRI studies showed no significant differences in diameter when measured by FSE or CE MRA (p = not significant). Clinically important abnormalities, such as aneurysm formation and restenosis, can be present years after treatment for aortic coarctation. In the regular follow-up of these patients, CE MRA may provide additional diagnostic information compared with FSE and should be included as part of the routine exam. Received: 3 April 2000; Revised: 5 July 2000; Accepted: 7 July 2000     

Spectrum of imaging findings in Takayasu arteritis—A case report

Takayasu arteritis (TA) is an uncommon chronic granulomatous inflammatory disease often affecting the aorta and its branches. Early diagnosis is quite challenging due to nonspecific symptoms and unfamiliarity with the disease. We hereby present a case of a young female patient diagnosed with Type V Takayasu arteritis using several radiological imaging modalities such as color and spectral Doppler study and computed tomography angiography. A timely diagnosis of Takayasu arteritis however may improve the outcome such as irreversible target organ damage and poor prognosis with a decreased rate of complications.     

Thoracic aorta: comparison of single-dose breath-hold and double-dose non-breath-hold gadolinium-enhanced three-dimensional MR angiography.

OBJECTIVE: The purpose of this study was to compare single-dose (0.1 mmol/kg) breath-hold gadolinium-enhanced three-dimensional (3D) MR angiography and double-dose (0.2 mmol/kg) non-breath-hold 3D MR angiography for evaluation of thoracic aortic disease. MATERIALS AND METHODS: Twenty-five patients referred for MR evaluation of the thoracic aorta underwent non-breath-hold gadolinium-enhanced 3D MR angiography on a 1.5-T scanner with standard gradients (TR/TE, 21/6; flip angle, 30 degrees) during slow infusion of a double dose of gadopentetate dimeglumine using a body coil. Subsequently, the same patients underwent breath-hold MR imaging with high-performance gradients (TR/TE, 5/2; flip angle, 30 degrees-50 degrees), a timing examination, and power injection of a single dose of gadolinium. For both studies, quantitative signal-to-noise measurements were obtained for the ascending thoracic, descending thoracic, and abdominal aorta. Three observers retrospectively evaluated each examination for degree of enhancement of the aorta, pulmonary arteries, and systemic veins; motion artifacts; and overall image quality. RESULTS: Single-dose breath-hold gadolinium-enhanced 3D MR angiography showed greater signal-to-noise ratio, fewer motion artifacts, and better overall image quality (p < .05) than the non-breath-hold double-dose technique. The single-dose technique also showed significantly better qualitative enhancement of the aortic root and ascending aorta (p < .05) and less enhancement of the pulmonary arteries, renal veins, and left internal jugular vein (p < .05). CONCLUSION: Optimized single-dose breath-hold gadolinium-enhanced 3D MR angiography is superior to double-dose non-breath-hold 3D MR angiography for evaluation of thoracic aortic disease.     

MR virtual angioscopy of thoracic aortic atherosclerosis in homozygous familial hypercholesterolemia

PURPOSE: The thoracic aorta is an important site of atherosclerotic disease in patients with homozygous familial hypercholesterolemia (HFH). Thoracic aortic atherosclerosis in patients with HFH was assessed with contrast-enhanced MR angiograms using exoscopic and endoscopic virtual angioscopy reconstructions and maximum intensity projections (MIPs). METHOD: Contrast-enhanced MR angiograms of the thoracic aorta of 15 patients with HFH and 8 normal volunteers were obtained. Perspective surface reconstructions of the MR angiograms including virtual angioscopy views were evaluated by three radiologists blinded to the diagnosis. RESULTS: Thoracic wall irregularity was depicted on 8 of 15 (53%) patient scans and only 1 of 8 (13%) normal subject scans using surface reconstructions. Wall irregularity scores of patients with HFH were significantly increased compared with controls (2.0 +/- 0.9 vs. 1.0 +/- 0.6; p = 0.008). There was excellent interobserver agreement (weighted kappa = 0.82 +/- 0.12). Virtual endoscopy views added diagnostic confidence compared with exoscopic surface renderings alone. MIP reconstructions were unable to depict wall irregularity. CONCLUSION: MR angiography with virtual angioscopy of the thoracic aorta depicts nonstenotic wall irregularity of thoracic aortic atherosclerosis in patients with HFH. This may be important for assessing disease progression and response to treatment and may be generalizable to routine (non-HFH) atherosclerosis.